• Amit Narang, Chandni Maheshwari, Varun Aggarwal, Puneet Bansal, and Paramdeep Singh.
• Department of Neurosurgery, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India.
• World Neurosurg. 2020 Jan 1; 133: 324-330.

BackgroundGorlin-Goltz syndrome is a rare hereditary disease affecting multiple organ systems. Medulloblastoma is the most common intracranial malignancy in these patients, radiotherapy makes them more susceptible to intracranial meningioma. Here we report an intracranial meningioma without radiation exposure.Case DescriptionWe present a case of intracranial meningioma in a young woman who was postoperatively diagnosed to have Gorlin-Goltz syndrome based on presence of calcification of bilateral tent and falx. Further clinical and radiological assessment helped us identify many other syndromic features and patient was promptly advised multispecialty consultations to screen for other malignancies and counselled regarding risk factors.ConclusionsEarly identification of the syndrome is important for prevention of secondary radiation-induced malignancies, both intracranial and extracranial. Patients need multidisciplinary approach for management.Copyright © 2019 Elsevier Inc. All rights reserved.

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